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Journal of Medical Case Reports Jun 2024Currently, there is a scarcity of cases and diagnostic data regarding ectopic adrenocortical adenomas, particularly in relation to their impact on gonadal function and... (Review)
Review
BACKGROUND
Currently, there is a scarcity of cases and diagnostic data regarding ectopic adrenocortical adenomas, particularly in relation to their impact on gonadal function and localization diagnostic techniques. We report a typical case of ectopic adrenocortical adenomas and the data of treatment follow-up, and review the literature of 31 available cases of ectopic adrenocortical adenomas.
CASE PRESENTATION
A 27-year-old Chinese female patient was admitted to our hospital for hypertension, hyperglycaemia and primary amenorrhea. The patient was functionally diagnosed with ACTH-independent CS and hypogonadotropic hypogonadism. Radiological evaluations, including Computed Tomography (CT) and functional imaging, identified a mass at the left renal hilum. Histological assessments post-surgical excision confirmed the mass to be an ectopic adrenocortical adenoma. A subsequent 3-month follow-up showed no signs of disease recurrence, a swift recovery of the cortisol axis was observed, with a partial recuperation of the gonadal axis.
REVIEW
Our literature review shows that the most common ectopic areas of cortisol adenomas are renal hilum and hepatic region. The most positive biomarker is Melan A, and only a few cases have been diagnosed with functional localization.
CONCLUSION
Ectopic adrenocortical adenomas may be asymptomatic in the early stage and can impact gonadal function. Physicians who treat hypogonadism must be aware of the need to test cortisol levels and perform functional localization in patients with lumps present.
Topics: Humans; Female; Adult; Hypogonadism; Adrenocortical Adenoma; Adrenal Cortex Neoplasms; Tomography, X-Ray Computed; Hydrocortisone
PubMed: 38863038
DOI: 10.1186/s13256-024-04595-z -
Diagnostic Pathology Jun 2024Inflammatory rhabdomyoblastic tumors are relatively recently recognized soft tissue tumors with a low malignant potential. Here, we present a case of concurrent...
BACKGROUND
Inflammatory rhabdomyoblastic tumors are relatively recently recognized soft tissue tumors with a low malignant potential. Here, we present a case of concurrent inflammatory rhabdomyoblastic tumor (IRMT), adrenal pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1 (NF1). To our knowledge, this is the first time that this constellation of tumors has been described in the literature.
CASE PRESENTATION
A female patient in her late 20s with known NF1 was diagnosed with an inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a short succession. IRMT was found to harbor a near-haploid genome and displayed a typical immunohistochemical profile as well as a focal aberrant p53 expression pattern.
CONCLUSIONS
This case report strengthens the theory that defects in the tumor suppressor NF1 play a central role in the pathogenesis of inflammatory rhabdomyoblastic tumors and that IRMT may be part of the spectrum of neurofibromatosis type 1 related tumors.
Topics: Humans; Neurofibromatosis 1; Female; Hamartoma; Pheochromocytoma; Adrenal Gland Neoplasms; Adult; Immunohistochemistry; Lung Diseases; Neurofibromin 1; Biomarkers, Tumor
PubMed: 38862977
DOI: 10.1186/s13000-024-01503-3 -
BMJ Case Reports Jun 2024The following case discusses the surgical considerations for a patient presenting with cardiogenic shock secondary to a phaeochromocytoma crisis with stress...
The following case discusses the surgical considerations for a patient presenting with cardiogenic shock secondary to a phaeochromocytoma crisis with stress cardiomyopathy. The patient underwent an interval laparoscopic adrenalectomy. Pneumoperitoneum insufflation was performed at lower pressures; manipulation of the adrenal tumour was minimised, and the adrenal vein was ligated early. However, as intraoperative blood pressure (BP) remained elevated and rising, further gentle dissection revealed an aberrant inferior phrenic vein draining the adrenal nodule. BP was finally reduced following ligation of the inferior phrenic vein, demonstrating the clinical significance of an unusual dual venous drainage from the adrenal nodule in this patient.
Topics: Humans; Pheochromocytoma; Adrenal Gland Neoplasms; Adrenalectomy; Takotsubo Cardiomyopathy; Female; Shock, Cardiogenic; Middle Aged; Laparoscopy; Perioperative Care; Male
PubMed: 38862185
DOI: 10.1136/bcr-2024-260479 -
European Clinical Respiratory Journal 2024Bronchoscopy and EBUS are standard procedures in lung cancer work-up but have low diagnostic yield in lesions outside the central airways and hilar/mediastinal lymph... (Review)
Review
BACKGROUND
Bronchoscopy and EBUS are standard procedures in lung cancer work-up but have low diagnostic yield in lesions outside the central airways and hilar/mediastinal lymph nodes. Growing evidence on introducing the EBUS endoscope into the oesophagus (EUS-B) in the same session as bronchoscopy/EBUS gives access to new anatomical areas that can be safely biopsied.
OBJECTIVE
To summarize the current evidence of the added value of EUS-B-FNA to bronchoscopy and EBUS-TBNA in lung cancer work-up.
METHODS
A narrative review.
RESULTS
Few randomized trials or prospective studies are available. Prospective studies show that add-on EUS-B-FNA increases diagnostic yield when sampling abnormal mediastinal lymph nodes, para-oesophageal lung and left adrenal gland. A large retrospective series on EUS-B-FNA from retroperitoneal lymph nodes suggests high diagnostic yield without safety concerns, as do casuistic reports on EUS-B-FNA from mediastinal pleural thickening, pancreatic lesions, ascites fluid and pericardial effusions. No study has systematically assessed both diagnostic yield, safety, patient reported outcomes, adverse events and costs.
CONCLUSION
The diagnostic value of add-on EUS-B to standard bronchoscopy and EBUS in lung cancer work-up appears very promising without safety concerns, giving the pulmonologist access to a variety of sites out of reach with other minimally invasive techniques. Little is known on patient-reported outcomes and costs. Future and prospective research should focus on effectiveness aspects to clarify whether overall benefits of add-on EUS-B sufficiently exceed overall downsides.
PubMed: 38859948
DOI: 10.1080/20018525.2024.2362995 -
BMJ Case Reports Jun 2024
Topics: Humans; Melanoma; Hemorrhage; Adrenal Gland Neoplasms; Skin Neoplasms; Male; Tomography, X-Ray Computed; Middle Aged; Adrenal Gland Diseases
PubMed: 38857916
DOI: 10.1136/bcr-2024-260227 -
Oncology Letters Aug 2024The aim of the present study was to develop and evaluate a clinical-imaging-radiomic nomogram based on pre-enhanced computed tomography (CT) for pre-operative...
Clinical‑imaging‑radiomic nomogram based on unenhanced CT effectively predicts adrenal metastases in patients with lung cancer with small hyperattenuating adrenal incidentalomas.
The aim of the present study was to develop and evaluate a clinical-imaging-radiomic nomogram based on pre-enhanced computed tomography (CT) for pre-operative differentiation lipid-poor adenomas (LPAs) from metastases in patients with lung cancer with small hyperattenuating adrenal incidentalomas (AIs). A total of 196 consecutive patients with lung cancer, who underwent initial chest or abdominal pre-enhanced CT scan with small hyperattenuating AIs, were included. The patients were randomly divided into a training cohort with 71 cases of LPAs and 66 cases of metastases, and a testing cohort with 31 cases of LPAs and 28 cases of metastases. Plain CT radiological and clinical features were evaluated, including sex, age, size, pre-enhanced CT value (CT), shape, homogeneity and border. A total of 1,316 radiomic features were extracted from the plain CT images of the AIs, and the significant features selected by the least absolute shrinkage and selection operator were used to establish a Radscore. Subsequently, a clinical-imaging-radiomic model was developed by multivariable logistic regression incorporating the Radscore with significant clinical and imaging features. This model was then presented as a nomogram. The performance of the nomogram was assessed by calibration curves and decision curve analysis (DCA). A total of 4 significant radiomic features were incorporated in the Radscore, which yielded notable area under the receiver operating characteristic curves (AUCs) of 0.920 in the training dataset and 0.888 in the testing dataset. The clinical-imaging-radiomic nomogram incorporating the Radscore, CT, sex and age revealed favourable differential diagnostic performance (AUC: Training, 0.968; testing, 0.915) and favourable calibration curves. The nomogram was revealed to be more useful than the Radscore and the clinical-imaging model in clinical practice by DCA. The clinical-imaging-radiomics nomogram based on initial plain CT images by integrating the Radscore and clinical-imaging factors provided a potential tool to effectively differentiate LPAs from metastases in patients with lung cancer with small hyperattenuating AIs.
PubMed: 38855505
DOI: 10.3892/ol.2024.14472 -
Acta Neurochirurgica Jun 2024During pituitary surgery, CSF leaks are often treated by intrasellar packing, using muscle or fat grafts. However, this strategy may interfere with the interpretation of...
BACKGROUND
During pituitary surgery, CSF leaks are often treated by intrasellar packing, using muscle or fat grafts. However, this strategy may interfere with the interpretation of postoperative MRI and may impact the quality of resection in cases of second surgery, due to the existence of additional fibrous tissue. We present an alternative technique, using a diaphragm reconstruction with a heterologous sponge combining fibrinogen and thrombin (TachoSil), applied in selected patients with low-flow CSF leaks. This study investigates the surgical outcome of patients treated with this strategy.
METHODS
From a cohort of 2231 patients treated from June 2011 to June 2023 by endoscopic endonasal approach for pituitary surgery, the surgical technique of diaphragm repair with TachoSil patch performed in 55 patients (2.6%) was detailed, and the rate of closure failure was analyzed at 6 months postoperatively. No intrasellar packing was used and sellar floor reconstruction was performed whenever possible. The rate of postoperative CSF leak was compared with that reported in three previous publications that also used the TachoSil patch technique.
RESULTS
Patients were mostly women (F/M ratio: 1.2) with a median age of 53.6 years. Surgery was indicated for non-functioning adenomas, Cushing's disease, acromegaly, and Rathke's cleft cysts in 38/55 (69.1%), 6/55 (10.9%), 5/55 (9.1%) and 6/55 (10.9%) patients respectively. The rate of postoperative CSF leak was 1.8% (n = 1/55), which was not significantly different from that reported in the three cohorts from the literature (2.8%, p > 0.05). No postoperative meningitis was recorded.
CONCLUSIONS
In highly selected patients with low-flow CSF leaks related to small focal diaphragm defects, diaphragm reconstruction using a TachoSil patch can be a safe and valuable alternative to intrasellar packing.
Topics: Humans; Female; Middle Aged; Thrombin; Male; Fibrinogen; Adult; Cerebrospinal Fluid Leak; Drug Combinations; Aged; Plastic Surgery Procedures; Cohort Studies; Diaphragm; Postoperative Complications; Pituitary Neoplasms; Treatment Outcome; Cerebrospinal Fluid Rhinorrhea; Pituitary Gland; Surgical Sponges
PubMed: 38853198
DOI: 10.1007/s00701-024-06152-5 -
BMJ Case Reports Jun 2024Adrenocortical tumours are rare in children and account for only 0.3%-0.4% of all neoplasms in childhood. They present with variable signs and symptoms, depending on the...
Adrenocortical tumours are rare in children and account for only 0.3%-0.4% of all neoplasms in childhood. They present with variable signs and symptoms, depending on the type of hormonal hypersecretion. The majority of the adrenocortical tumours in children are functional (90%) and malignant (88%). Here, we describe a functional plurihormonal oncocytic adrenal cortical adenoma in a young girl, that mimicked a malignant adrenal lesion, clinically as well as on imaging and biochemical features. This report bears the objective of being aware of the atypical biochemical as well as imaging characteristics of oncocytic adrenal tumours.
Topics: Female; Humans; Adenoma, Oxyphilic; Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Diagnosis, Differential; Tomography, X-Ray Computed; Adolescent
PubMed: 38851224
DOI: 10.1136/bcr-2023-259327 -
Therapeutic Advances in Medical Oncology 2024Perioperative use of immune checkpoint blockade (ICB) improves survival in patients with early-stage cancer. Treatment-related adverse events (AEs), frequently involve...
BACKGROUND
Perioperative use of immune checkpoint blockade (ICB) improves survival in patients with early-stage cancer. Treatment-related adverse events (AEs), frequently involve the endocrine system which may increase perioperative complications and affect quality of life.
OBJECTIVE
We conducted a meta-analysis to elucidate the impact of adding ICB to conventional neoadjuvant/adjuvant therapy on the incidence of endocrine AEs.
DESIGN
A systematic review and meta-analysis of randomize-controlled trials (RCTs).
DATA SOURCES AND METHODS
A systematic search of PubMed, Embase, Web of Science, and Cochrane library was performed for RCTs comparing groups with and without the addition of ICB to conventional perioperative therapy in patients with cancer. Outcomes included all-grade and grade 3-5 thyroiditis, hyperthyroidism, hypothyroidism, adrenal insufficiency, hypophysitis, type 1 diabetes mellitus, and hyperglycemia. The odds ratios (ORs) of all-grade and grade 3-5 endocrine were pooled using the random-effect model meta-analysis.
RESULTS
Twenty-four RCTs comprising 12,199 patients were identified for meta-analysis. The addition of ICB was associated with higher incidence of thyroiditis [all grade: OR = 3.53 (95% confidence interval (CI): 1.88-6.64)], hyperthyroidism [all-grade: 7.18 (4.30-12.01); grade 3-5: 3.93 (1.21-12.82)], hypothyroidism [all-grade: 5.39 (3.68-7.90); grade 3-5: 3.63 (1.18-11.11)], adrenal insufficiency [all-grade: 3.82 (1.88-7.79); grade 3-5: 5.91 (2.36-14.82)], hypophysitis [all-grade: 10.29 (4.97-21.3); grade 3-5: 5.80 (1.99-16.92)], and type 1 diabetes mellitus [all-grade: 2.24 (1.06-4.74); grade 3-5: 3.49 (1.21-10.08)]. The cumulative incidence of each grade 3-5 endocrine AE was low (<1.3%). No grade 5 AEs leading to death were observed.
CONCLUSION
The addition of neoadjuvant/adjuvant ICB to conventional therapy was associated with an increased incidence of several endocrine AEs. Clinicians should be aware of the risk of endocrinopathy from the perioperative ICB use to facilitate risk-benefit discussion with patients with early-stage cancer.
TRIAL REGISTRATION
The protocol of this research was registered in PROSPERO (CRD42022332624).
PubMed: 38845790
DOI: 10.1177/17588359241257874 -
Surgical Neurology International 2024Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and...
BACKGROUND
Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor.
CASE DESCRIPTION
An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months.
CONCLUSION
This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.
PubMed: 38840611
DOI: 10.25259/SNI_116_2024